Comprehensive description of the prevalence, serological and clinical characteristics, and visceral involvement of systemic sclerosis (scleroderma) in a large cohort from the United Arab Emirates Systemic Sclerosis Registry.

Systemic sclerosis is an autoimmune condition characterized by a wide range of clinical presentations. Registries may serve to expand understanding about systemic sclerosis and aid in patient care and follow-up. The objective of this study was to analyze the prevalence of systemic sclerosis in a large cohort from the United Arab Emirates Systemic Sclerosis Registry and find the significant similarities and differences between the different subsets. All scleroderma patients in the United Arab Emirates were included in this multicenter national retrospective analysis. Data on demographics, comorbidities, serological characteristics, clinical aspects, and treatment were collected and analyzed, highlighting the most common traits identified. A total of 167 systemic scleroderma patients from diverse ethnic backgrounds were enrolled. Overall, 54.5% (91/167) of the patients were diagnosed with diffuse cutaneous systemic sclerosis, and 45.5% (76/167) with limited cutaneous systemic sclerosis. The prevalence of systemic sclerosis was 1.66 per 100,000 for the total registry and 7.78 per 100,000 for United Arab Emirates patients. Almost all patients in the diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis groups tested positive for the immunofluorescence antinuclear antibody. Antibodies against Scl-70 were significantly more associated with diffuse cutaneous systemic sclerosis, whereas anticentromere antibodies were significantly more associated with the limited cutaneous systemic sclerosis group (p < 0.001). Sclerodactyly, shortness of breath, and digital ulcers were more common in diffuse cutaneous systemic sclerosis patients compared with the limited cutaneous systemic sclerosis subtype in terms of clinical symptoms and organ involvement. Telangiectasia was much more common in the limited cutaneous systemic sclerosis group. Furthermore, diffuse cutaneous systemic sclerosis patients had more lung fibrosis (interstitial lung disease) than limited cutaneous systemic sclerosis patients (70.5% vs 45.7%), and pulmonary arterial hypertension was twice as common in limited cutaneous systemic sclerosis patients as it was in diffuse cutaneous systemic sclerosis patients. Local registries are paramount to understanding the clinical/serological characteristics of scleroderma. This study emphasizes the importance of raising disease awareness and distinguishing between the various systemic sclerosis subsets to implement patient-tailored strategies for early detection, better management, and higher quality of care.

Outpatient Care in Psychiatry for Transitional Age Youth (16 to 24 Years Old): Which Trajectories of Psychiatric Care in French-Speaking Belgium?

BACKGROUND: Transitional age youth (TAY) are a particularly at-risk population in mental health. They have specific needs, not currently covered between child and adolescent mental health services (CAMHS) and adult mental health services (AMHS), mainly because of existing barriers. This retrospective study was carried out to describe sociodemographic and clinical characteristics of patients who attended a new outpatient program specifically conceived for TAY in Brussels, Belgium. Outcomes related to trajectories of psychiatric care were analysed, such as leading symptom, consultation's referral and requester, and final orientation. SUBJECTS AND METHODS: The total sample included 243 patients aged from 16 to 24 years old who presented for care in this outpatient program between October 2019 and May 2021. Data were retrospectively extracted from each patient's electronic medical records. RESULTS: The sample was mainly composed by female participants; the average age was 18.7 (±2.0) years. General practitioner (18.9%), child and adolescent psychiatrist (18.1%), psychologist (11.5%) and adult psychiatrist (7.4%) referred patients. Leading symptoms were divided into three dimensions: internalizing (67.5%), externalizing (21.8%) and psychotic (10.7%) ones. After first assessment, 81.5% of youth were followed-up in our specific outpatient program. Overall, at the end of data collection, youth were orientated towards ongoing follow-up in this program (37%), AMHS care (21.8%), end of psychiatric care (17.3%) and CAMHS care (4.1%). 19.8% patients discontinued the proposed care. CONCLUSIONS: This psychiatric outpatient program for TAY represents an innovative contribution to reinforce CAMHS-AMHS interface in French-speaking Belgium. The analysis of trajectories in psychiatric care suggests positive outcomes of this TAY-tailored clinical program to achieve high quality standard of care in youth mental health. Future research and clinical implementation programs should further explore which factors influence decision-making process in TAY psychiatric assessment and care.

Nano-Curcumin Protects Against Sodium Nitrite-Induced Lung Hypoxia Through Modulation of Mitogen-Activated Protein Kinases/c-Jun NH2-Terminal Kinase Signaling Pathway.

BACKGROUND AND OBJECTIVE: This study was designed to compare the efficacy of curcumin (CRN) with that of nano-curcumin (N-CRN) in the mitigation of various biochemical indices in hypoxic lung induced by sodium nitrite (SN) in rats. METHODS: Twenty-four adult male albino rats were divided into 4 groups. Group 1: control group received carboxy methyl cellulose; Group 2: hypoxic group injected with single dose of SN (60 mg/kg, s.c.); Group 3: SN-intoxicated rats pre-injected with CRN (100 mg/kg, i.p.); and Group 4: SN-intoxicated rats pre-injected with N-CRN (100 mg/kg, i.p.). Curcumin and N-CRN were administered intraperitoneally 2 hour prior to SN intoxication. Hemoglobin concentration, serum tumor necrosis factor-alpha (TNF-α), and caspase-3 were analyzed. Gene expression of hypoxia inducible factor-1 (HIF-1α), matrix metallo-proteinases (MMP)-2, and tissue inhibitors of metalloproteinases (TIMPs)-2, as well as the protein expression of mitogen-activated protein kinases (MAPKs) and c-Jun NH2-terminal kinase (JNK) were examined in lung tissues. RESULTS: Hemoglobin level was markedly reduced, and serum TNF-α and caspase-3 were significantly elevated post SN intoxication. The lung MMP-2 and HIF-1α mRNA were overexpressed in the hypoxic group; while TIMP-2 mRNA was downregulated. Sodium nitrite administration increased proteins' expressions of MAPK and JNK. Pretreatment with CRN or N-CRN markedly mitigated those alterations. These results were supported by histopathological examinations of lung tissue. CONCLUSION: Interestingly, N-CRN exhibited a pronounced protective effect via suppression of inflammatory and apoptotic biomarkers and modulation of MAPK/JNK signaling pathway.

Deep Brain Stimulation in KMT2B-Related Dystonia: Case Report and Review of the Literature With Special Emphasis on Dysarthria and Speech.

Objective: KMT2B-related dystonia is a progressive childhood-onset movement disorder, evolving from lower-limb focal dystonia into generalized dystonia. With increasing age, children frequently show prominent laryngeal or facial dystonia manifesting in dysarthria. Bilateral deep brain stimulation of the globus pallidus internus (GPi-DBS) is reported to be an efficient therapeutic option. Especially improvement of dystonia and regaining of independent mobility is commonly described, but detailed information about the impact of GPi-DBS on dysarthria and speech is scarce. Methods: We report the 16-months outcome after bilateral GPi-DBS in an 8-year-old child with KMT2B-related dystonia caused by a de-novo c.3043C>T (p.Arg1015*) non-sense variant with special emphasis on dysarthria and speech. We compare the outcome of our patient with 59 patients identified through a PubMed literature search. Results: A remarkable improvement of voice, articulation, respiration and prosodic characteristics was seen 16 months after GPi-DBS. The patients' speech intelligibility improved. His speech became much more comprehensible not only for his parents, but also for others. Furthermore, his vocabulary and the possibility to express his feelings and wants expanded considerably. Conclusion: A positive outcome of GPi-DBS on speech and dysarthria is rarely described in the literature. This might be due to disease progression, non-effectiveness of DBS or due to inadvertent spreading of the electrical current to the corticobulbar tract causing stimulation induced dysarthria. This highlights the importance of optimal lead placement, the possibility of horizontal steering of the electrical field by applying directional stimulation with segmented leads as well as the use of the lowest possible effective stimulation intensity.

Evaluation of noninvasive versus invasive techniques for the diagnosis of Helicobacter pylori infection.

BACKGROUND: Helicobacter pylori is one of the most common bacterial strains causing chronic infections, affecting over one half of the world's population. There is increasing interest in noninvasive methods for diagnosing H. pylori infection. The aim of the study was to evaluate 3 different noninvasive methods of diagnosis: the stool antigen test (HpSA), the serum antibody test, and the stool-polymerase chain reaction (PCR) test as against invasive methods based on histopathologic diagnosis. MATERIALS AND METHODS: Gastric biopsies were obtained during endoscopy. Sections were stained with hematoxylin and eosin and Giemsa stain. Serum samples were tested for H. pylori antibody using an enzyme-linked immnunosorbent assay kit for the semiquantitative determination of IgG antibodies; stool samples were tested for H. pylori antigen using polyclonal enzyme-linked immnunosorbent assay kits. DNA samples from stool specimens were extracted, followed by PCR for the detection of H. pylori UreA. RESULTS: The results revealed that 18/19 (94.7%) patients were positive for H. pylori infection as detected by Giemsa stain, and 84.2% were positive on the basis of hematoxylin and eosin stain, with a sensitivity and specificity of 88.9% and 100%, respectively. Diagnosis by noninvasive methods, including the serum antibody test, revealed a sensitivity and positive predictive value of 88.9% and 94.2%, respectively, whereas the stool antigen test recorded a sensitivity and positive predictive value of 72.2% and 92.9%, respectively. The stool-PCR test recorded a sensitivity of 72.2% and specificity of 100%. CONCLUSIONS: Among the noninvasive methods for diagnosis of H. pylori infection, the 3 methods used in this study recorded promising results, including good sensitivity, which was the highest in the serum antibody test, whereas the stool-PCR test recorded excellent specificity.